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Pulmonary langerhans cell histiocytosis
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular...
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description | Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation. |
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The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.</description><identifier>ISSN: 1750-1172</identifier><identifier>EISSN: 1750-1172</identifier><identifier>DOI: 10.1186/1750-1172-7-16</identifier><identifier>PMID: 22429393</identifier><language>eng</language><publisher>England: BioMed Central Ltd</publisher><subject>Adult ; Bronchiolitis ; Care and treatment ; Chemotherapy ; Child, Preschool ; Cigarette smoke ; Cigarettes ; Development and progression ; Diagnosis ; Disease ; Female ; Health aspects ; Histiocytosis, Langerhans-Cell - complications ; Histiocytosis, Langerhans-Cell - epidemiology ; Histiocytosis, Langerhans-Cell - immunology ; Histiocytosis, Langerhans-Cell - pathology ; Humans ; Hypertension ; Hypertension, Pulmonary - complications ; Infant ; Interstitial ; Langerhans cells ; Langerhans Cells - immunology ; Langerhans Cells - pathology ; Langerhans-cell histiocytosis ; Lung diseases ; Lung Diseases - complications ; Lung Diseases - epidemiology ; Lung Diseases - immunology ; Lung Diseases - pathology ; Male ; Medical research ; Pathogenesis ; Pneumothorax - complications ; Prognosis ; Pulmonary hypertension ; Rare diseases ; Recruitment ; Respiratory Function Tests ; Review ; Risk factors ; Smokers ; Smoking ; Smoking - adverse effects ; Tobacco ; Young Adult</subject><ispartof>Orphanet journal of rare diseases, 2012-03, Vol.7 (1), p.16-16, Article 16</ispartof><rights>COPYRIGHT 2012 BioMed Central Ltd.</rights><rights>2012 Suri et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</rights><rights>Copyright ©2012 Suri et al; licensee BioMed Central Ltd. 2012 Suri et al; licensee BioMed Central Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b779t-2eea2d319507f96d7c0589f48b48caed5ff0ad59a6a3e84a28930831e378b0703</citedby><cites>FETCH-LOGICAL-b779t-2eea2d319507f96d7c0589f48b48caed5ff0ad59a6a3e84a28930831e378b0703</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3342091/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.proquest.com/docview/1010625256?pq-origsite=primo$$EHTML$$P50$$Gproquest$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,881,25732,27903,27904,36991,36992,44569,53770,53772</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22429393$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Suri, Harpreet S</creatorcontrib><creatorcontrib>Yi, Eunhee S</creatorcontrib><creatorcontrib>Nowakowski, Gregorz S</creatorcontrib><creatorcontrib>Vassallo, Robert</creatorcontrib><title>Pulmonary langerhans cell histiocytosis</title><title>Orphanet journal of rare diseases</title><addtitle>Orphanet J Rare Dis</addtitle><description>Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.</description><subject>Adult</subject><subject>Bronchiolitis</subject><subject>Care and treatment</subject><subject>Chemotherapy</subject><subject>Child, Preschool</subject><subject>Cigarette smoke</subject><subject>Cigarettes</subject><subject>Development and progression</subject><subject>Diagnosis</subject><subject>Disease</subject><subject>Female</subject><subject>Health aspects</subject><subject>Histiocytosis, Langerhans-Cell - complications</subject><subject>Histiocytosis, Langerhans-Cell - epidemiology</subject><subject>Histiocytosis, Langerhans-Cell - immunology</subject><subject>Histiocytosis, Langerhans-Cell - pathology</subject><subject>Humans</subject><subject>Hypertension</subject><subject>Hypertension, Pulmonary - complications</subject><subject>Infant</subject><subject>Interstitial</subject><subject>Langerhans cells</subject><subject>Langerhans Cells - immunology</subject><subject>Langerhans Cells - pathology</subject><subject>Langerhans-cell histiocytosis</subject><subject>Lung diseases</subject><subject>Lung Diseases - complications</subject><subject>Lung Diseases - epidemiology</subject><subject>Lung Diseases - immunology</subject><subject>Lung Diseases - pathology</subject><subject>Male</subject><subject>Medical research</subject><subject>Pathogenesis</subject><subject>Pneumothorax - complications</subject><subject>Prognosis</subject><subject>Pulmonary hypertension</subject><subject>Rare diseases</subject><subject>Recruitment</subject><subject>Respiratory Function Tests</subject><subject>Review</subject><subject>Risk factors</subject><subject>Smokers</subject><subject>Smoking</subject><subject>Smoking - adverse effects</subject><subject>Tobacco</subject><subject>Young Adult</subject><issn>1750-1172</issn><issn>1750-1172</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>PIMPY</sourceid><sourceid>DOA</sourceid><recordid>eNp1kk1v1DAQhiMEoqVw5YhW4kA5pPgrsX2ptKoKrFQJxMfZmjh21qvELnaC6L_HYcvSQJEPtmZePzN-x0XxHKMzjEX9BvMKlRhzUvIS1w-K40Pg4Z3zUfEkpR1CrKJIPC6OCGFEUkmPi1cfp34IHuLNqgffmbgFn1ba9P1q69Logr4ZQ3LpafHIQp_Ms9v9pPj69vLLxfvy6sO7zcX6qmw4l2NJjAHSUiwrxK2sW65RJaRlomFCg2kraxG0lYQaqBEMiJC5I4oN5aJBHNGTYrPntgF26jq6IbemAjj1KxBipyCOTvdGIZbrUGGN5oxhhKTFrNU5YC0whiCzzves66kZTKuNHyP0C-gy491WdeG7opQRJHEGrPeAxoX_AJYZHQY1m65m0xVXuM6M09smYvg2mTSqwaXZX_AmTElhhOdBEimy9OVf0l2Yos92zypUk4pU9R9VB9kE523IpfUMVWsiBKNEMJlVZ_eo8mrN4HTwxrocX1x4vbiQNaP5MXYwpaQ2nz_dC9cxpBSNPViC56eL-l8TXtydxEH--x_Sn3pb2bk</recordid><startdate>20120319</startdate><enddate>20120319</enddate><creator>Suri, Harpreet S</creator><creator>Yi, Eunhee S</creator><creator>Nowakowski, Gregorz S</creator><creator>Vassallo, Robert</creator><general>BioMed Central Ltd</general><general>BioMed Central</general><general>BMC</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>ISR</scope><scope>3V.</scope><scope>7T5</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AN0</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20120319</creationdate><title>Pulmonary langerhans cell histiocytosis</title><author>Suri, Harpreet S ; Yi, Eunhee S ; Nowakowski, Gregorz S ; Vassallo, Robert</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b779t-2eea2d319507f96d7c0589f48b48caed5ff0ad59a6a3e84a28930831e378b0703</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adult</topic><topic>Bronchiolitis</topic><topic>Care and treatment</topic><topic>Chemotherapy</topic><topic>Child, Preschool</topic><topic>Cigarette smoke</topic><topic>Cigarettes</topic><topic>Development and progression</topic><topic>Diagnosis</topic><topic>Disease</topic><topic>Female</topic><topic>Health aspects</topic><topic>Histiocytosis, Langerhans-Cell - complications</topic><topic>Histiocytosis, Langerhans-Cell - epidemiology</topic><topic>Histiocytosis, Langerhans-Cell - immunology</topic><topic>Histiocytosis, Langerhans-Cell - pathology</topic><topic>Humans</topic><topic>Hypertension</topic><topic>Hypertension, Pulmonary - complications</topic><topic>Infant</topic><topic>Interstitial</topic><topic>Langerhans cells</topic><topic>Langerhans Cells - immunology</topic><topic>Langerhans Cells - pathology</topic><topic>Langerhans-cell histiocytosis</topic><topic>Lung diseases</topic><topic>Lung Diseases - complications</topic><topic>Lung Diseases - epidemiology</topic><topic>Lung Diseases - immunology</topic><topic>Lung Diseases - pathology</topic><topic>Male</topic><topic>Medical research</topic><topic>Pathogenesis</topic><topic>Pneumothorax - complications</topic><topic>Prognosis</topic><topic>Pulmonary hypertension</topic><topic>Rare diseases</topic><topic>Recruitment</topic><topic>Respiratory Function Tests</topic><topic>Review</topic><topic>Risk factors</topic><topic>Smokers</topic><topic>Smoking</topic><topic>Smoking - adverse effects</topic><topic>Tobacco</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Suri, Harpreet S</creatorcontrib><creatorcontrib>Yi, Eunhee S</creatorcontrib><creatorcontrib>Nowakowski, Gregorz S</creatorcontrib><creatorcontrib>Vassallo, Robert</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: Science</collection><collection>ProQuest Central (Corporate)</collection><collection>Immunology Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central UK/Ireland</collection><collection>British Nursing Database</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Publicly Available Content (ProQuest)</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>Orphanet journal of rare diseases</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Suri, Harpreet S</au><au>Yi, Eunhee S</au><au>Nowakowski, Gregorz S</au><au>Vassallo, Robert</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pulmonary langerhans cell histiocytosis</atitle><jtitle>Orphanet journal of rare diseases</jtitle><addtitle>Orphanet J Rare Dis</addtitle><date>2012-03-19</date><risdate>2012</risdate><volume>7</volume><issue>1</issue><spage>16</spage><epage>16</epage><pages>16-16</pages><artnum>16</artnum><issn>1750-1172</issn><eissn>1750-1172</eissn><abstract>Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.</abstract><cop>England</cop><pub>BioMed Central Ltd</pub><pmid>22429393</pmid><doi>10.1186/1750-1172-7-16</doi><oa>free_for_read</oa></addata></record> |
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subjects | Adult Bronchiolitis Care and treatment Chemotherapy Child, Preschool Cigarette smoke Cigarettes Development and progression Diagnosis Disease Female Health aspects Histiocytosis, Langerhans-Cell - complications Histiocytosis, Langerhans-Cell - epidemiology Histiocytosis, Langerhans-Cell - immunology Histiocytosis, Langerhans-Cell - pathology Humans Hypertension Hypertension, Pulmonary - complications Infant Interstitial Langerhans cells Langerhans Cells - immunology Langerhans Cells - pathology Langerhans-cell histiocytosis Lung diseases Lung Diseases - complications Lung Diseases - epidemiology Lung Diseases - immunology Lung Diseases - pathology Male Medical research Pathogenesis Pneumothorax - complications Prognosis Pulmonary hypertension Rare diseases Recruitment Respiratory Function Tests Review Risk factors Smokers Smoking Smoking - adverse effects Tobacco Young Adult |
title | Pulmonary langerhans cell histiocytosis |
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